Recombinant Human GCSH protein (Catalog#14568-H07E)
Preparation
Produced in rabbits immunized with purified, recombinant Human GCSH (rh GCSH; Catalog#14568-H07E; AAA36011.1; Ser49-Glu173). Total IgG was purified by Protein A affinity chromatography.
Source
Polyclonal Rabbit IgG
Purification
Protein A
Formulation
0.2 μm filtered solution in PBS
Conjugate
Unconjugated
Form
Liquid
Shipping
This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
Storage
This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free. Avoid repeated freeze-thaw cycles.
glycine cleavage system protein H (aminomethyl carrier)
Description
Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). GCSH is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in GCSH gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding,have been found for GCSH gene. Also, several transcribed and non-transcribed pseudogenes of GCSH gene exist throughout the genome.
References
Hiraga K. et al., 1988, Biochem Biophys Res Commun. 151 (2): 758-62.
Fujiwara K. et al., 1991, Biochem Biophys Res Commun. 176 (2): 711-6.
Koyata H. et al., 1991, Am J Hum Genet. 48 (2): 351-61.